DISORDERS OF HEMATOLOGICAL SYSTEM BY- RAMYA DEEPTHI P ASST PROFESSOR VIJAY

MARIE COLLGE OF NURSING

DISORDERS OF BLOOD A. ANEMIA B. THALASSEMIA C. HEMOPHILIA D. LEUKEMIA E. .2

PURPURA

ANEMIA .3

Definition Anemia is defined as reduction in the volume of red blood cells or in- .4
concentration of hemoglobin, below the lower limit of the normal range for age and sex of
the individual

CLASSIFICATION Two widely accepted classification of anemia are- I. Classification based .5

on Morphology II. Classification based on Etiology

BASED ON MORPHOLOGY Based on mean corpuscular volume, anemia is of three types- .6

1. Microcytic : abnormally small RBC’s are present in iron deficiency anemia and certain non
iron deficiency anemia's like sideroblastic anemia and thalassemia. 2. Normocytic anemia :
RBC’s are normal in shape but anemia occurs due to blood loss, Hemolysis or bone marrow
failure. 3. Macrocytic anemia : in this type, the RBC’s are abnormally large in shape. It is
.usually due to vit-B12 or folic acid deficiency

Anemia due to impaired red cell production a. Iron deficiency anemia b. Vit B12 and .2 .7
folic acid deficiency anemia c. Aplastic anemia d. Aplasia of pure red blood cells e. Anemia
due to infection f. Anemia in renal disease g. Anemia in liver disease h. Anemia in
disseminated malignancy i. Anemia in endocrinopathies j. Leukemia k. Myelosclerosis l.
Multiple myeloma m. Sickle cell anemia 1. Anemia due to blood loss a. Acute post
hemorrhagic anemia b. Chronic post hemorrhagic anemia 2. Anemia due to increases
destruction of red blood cells A. Anemia due to intracorpuscular defect a) Sickle cell anemia
b) Thalassemia B. Anemia due to extracorpuscular defect a) Hemolytic disease of new born
b) Effect of toxic drugs c) Effect of venoms or poisoning from substances like lead d) Thermal
injury or burns e) Transfusion reactions f) Infections like infectious mononucleosis BASED ON
ETIOLOGY

Diagnosis of anemia Anemia can be diagnosed based on the basis of – 1. Hemoglobin .8

estimation 2. Peripheral blood film examination I. Variation in size of RBC’s II. Variation in
shape of RBC’s III. Inadequate hemoglobin content 3. Red cell indices 4. Leucocyte,
reticulocyte and platelet count 5. Bone marrow aspiration

I Hemoglobin estimation If the hemoglobin amount is less than the expected normal .9
.range and sex then the person is said to be anemic

II Peripheral blood film examination Examination of a well prepared and stained .10
peripheral blood smear is most helpful in diagnosis of anemia. The following abnormalities
can be seen in the blood smear- i. Variation in size of RBC’s: normally the diameter of RBC’s
is 6.7 – 7.7 um. Increased variation in size of the RBC’s is termed as Variation in shape of
‘Anisocytosis’ ii. Variation in shape of RBC’s: increased variation in shape of RBC’s is known
as ‘Poikilocytosis’. It is seen in megaloblastic anemia, thalassemia, iron deficiency anemia. iii.
Inadequate hemoglobin content: normally on staining red cells, they appear pink in color.
The intensity of pink color depends on the level of HB present. A low Hb Content may be low
in iron content known as ‘Hypochromasia’ and increases content known as Hyperchromasia

III Red cell indices Measurement of red cell indices help to diagnose the type and .11
severity of anemia. In iron deficiency anemia MCV, MCH, MCHC are reduced. In
megaloblastic anemia MCV is more than the normal range. MCV- mean corpuscular volume
MCHC mean corpuscular hemoglobin concentration MCH- mean corpuscular hemoglobin

IV Leucocytes, Reticulocyte and platelet count 1. Estimation of leucocyte and PC helps in .12
distinguishing pure anemia from pancytopenia, in which red cells, granulocytes and platelets
all are recorded. 2. In anemia due to hemolysis or blood loss, neutrophil and PC are
elevated. 3. In infection, leukemia, leucocytes counts are elevated and immatutre leucocytes
appears in blood. 4. After acute hemorrhage or hemolysis, reticulocyte count rises with in 2-
.3 days. 5. An impaired reticulocyte count indicates impaired bone marrow function

Bone marrow examination Bone marrow aspiration may be performed because cellular .13
.changes with in marrow are diagnostic of many hematological conditions like leukemia

RON DEFICIENCY ANEMIA .14

Introduction • Iron deficiency anemia is the most common hematological disorder of .15
infancy and childhood. • It is caused by lack of iron for the synthesis of hemoglobin

Iron absorption .16

Iron absorption and metabolism • Iron required for Hb synthesis is derived from two .16
sources- ingestion of food rich in iron, recycling of iron form broken RBC’s. • Dietary form is
absorbed in the small intestine and either passed into blood stream or stored in intestinal
epithelial cells as ferritin. • The iron in blood stream binds to iron- transport molecule-
transferritin • Then it is delivered to RBC’s in bone marrow • It combines with other
.components of hemoglobin

ETIOLOGY SEVERAL FACTORS MAY CONTRIBUTE TO IRON DEFICIENCY ANEMIA .17

INCLUDINH- 1. Increased blood loss: peptic ulcer, polyps, hematuria, parasitic infestations
and epistaxis. 2. Insufficient iron supply at birth: infants born to anemic mothers receive
inadequate iron form mother during IU life. Also, if baby is preterm or had lost blood before
.or during process of birth, he is prone to iron deficiency anemia

Contd… 3. Insufficient iron intake: maternal supply of iron is sufficient for first 4-5 .18
months of infants life. Maternal insufficiency of iron to baby will lead to development of
anemia with in 2 months of life. Infant fed on cow’s milk should be given iron
supplementation as it is poor in iron. 4. Impaired iron absorption: mal absorption syndrome,
.chronic diarrhea, intake of antacids and tea after meals

Pathophysiology • Due to any etiological factor, when sufficient iron is not available for .19
hemoglobin synthesis, the production of hemoglobin is decreased. • As the hemoglobin
decreases, the newly formed RBC’s become smaller (Microcytic) and less filled with
hemoglobin (Hypochromic) • This results in decreased Hb levels and reduced oxygen
carrying capacity of blood

Contd.. Development of anemia progresses in 3 stages. 1. Firstly, iron stores are .20
depleted in an attempt to supply iron for erythropoiesis. 2. In the second step, iron supply
for erythropoiesis is reduced without development of anemia. 3. The final step is
development of frank anemia with Microcytic and Hypochromic RBC’s

CLINICAL FEATURES • The most clinical feature of anemia is PALLOR. • When Hb levels .21
falls 5-6 gm/dl, following features are developed in child- i. Irritability ii. Constipation iii.
Cardiac enlargement iv. Tachycardia v. weakness vi. Dyspnea vii. Poor attention viii. Reduced
alertness

Diagnostic evaluation Anemia may be mild, moderate, severe. It can be diagnosed on .22
the following basis- I. History of child II. Blood test III. Peripheral blood smear IV. Stool test

i. History of the child • a through dietary history is essential in making diagnosis of iron .23
deficiency of anemia. • The history usually reveals high milk intake and low intake of iron
.containing foods

ii. Blood test • Hemoglobin level is below 11gm/dl • Hematocrit is below 33% • MCV is .24
bellow 70um3 in infants and below 75 um3 in children • Reticulocyte count is reduced •
Serum ferritin concentration is below 10 mg/dl • Serum iron value is below 30 ug/dl • Total
iron binding capacity is elevated to 350 um/ dl in an attempt to absorb more iron

iii. Peripheral blood smear • The smear shows microcytric and hypochromic red cells .25
.which may vary in shape (poikilocytosis) and size (anisocytosis)

iv. Stool test • Stool is tested for presence of occult blood which indicates bleeding form .26
.gastrointestinal tract

MANAGEMENT I. Oral iron therapy II. Parenteral iron therapy III. Blood transfusion .27

I. ORAL IRON THERAPY • A therapeutic dose of 6mg/kg/day of elemental iron given .28
orally in 3 divided doses provide an optional amount of iron needed for hemoglobin
synthesis. • With this dose, the hemoglobin level should rise by 0.4gm/dl per day. • Oral iron
therapy should be continued for at least 6-8 weeks after the hemoglobin has reached normal
.level

II. PARENTERAL IRON THERAPY • A parenteral iron preparation, iron dextran which .29
contains 50mg elemental iron per ml is used, when therapeutic result of oral iron therapy is
not achieved. • Iron requirement of the body is determined by the following equation- Iron
requirement (mg)=wt (kg) X Hb deficit (g/dl) X 4 • Daily dose of iron dextran should be
limited to 50 mg in infants and 100 mg in adults. • Ity is administered intramuscularly using
Z-tract method, deeply into large muscle mass. • Side effects of parenteral iron include pain,
chills, fever, arthralgia, shock and even fatal anaphylaxis

III. BLOOD TRANSFUSION • When anemia is severe , the child may go into congestive .30
heart failure. • When the hemoglobin levels is below 4gm/dl, only packed cells should be
given slowly. • Also one or two doses of frusemide 1-2mg/kg, intravenously should be given
.to prevent circulatory overload

NURSING MANAGEMENT • Nursing management focuses on parental education, It .31

consists of Teaching about - 1. proper administration of iron supplements 2. Side effects of
iron therapy 3. Improving dietary iron intake

i. proper administration of iron supplements • Iron medication should be given between .32
meals • Medication should not be administered with milk or tea which reduces its
absorption • It should be given with any form of ascorbic acid such as citrus fruit or juice
which aids in absorption • Liquid iron preparation can stain teeth temporarily so it should be
given to infants with medicine dropper or syringe placed towards the back of the mouth •
Older teeth can take this solution through straw followed by rinsing mouth or brushing

ii. Side effects of iron therapy • Abdominal cramps • Nausea • Vomiting • Diarrhoea or .33
constipation • Should be given with meals to prevent gastrointestinal irritation

iii. Improving dietary iron intake • Iron rich foods-  Meat  Liver  Kidney  Egg yolk  .34
Green leafy vegetables  Fruits like apple  At the time of weaning iron rich diet should be
given to infant  Food should be prepared in utensils made up of iron, to increase the iron
.content of food

iv. prevention • Imp responsibility is to educate parents on prevention.  Iron fortified .35
milk formulas should be used for non-breast fed infants.  Prevent worm infestations 
Antihelmenthic drugs to manage infestations  Cook food in iron utensils.  Iron
supplements should be administered to preterm and low birth weight infants having low
.iron stores

MEGALOBLASTIC ANEMIA .36

INTRODUCTION • When deficiency of vitamin B12 or folic acid occurs, the rate of DNA .37
and RNA synthesis is reduced, delaying cell division. The cells thus get extra time between
divisions so they grow larger than normal resulting in formation of Megaloblasts. •
Deficiency of vitamin B12 and folic acid impairs the maturation of erythrocytes leading to
.formation of abnormally large erythrocytes known as Megaloblasts

ETIOLOGY • Results from deficiency of vitamin B12 and folic acid which may occur in .38
following conditions-  Inadequate dietary intake of vit B12 and Folic acid  Mal-absorption 
Treatment with anticonvulsants  Chemotherapy  Excess urinary folate loss ( active liver
disease and congestive heart failure)  Intrinsic defect of folic acid absorption

Clinical features • Pallor • Sick look • Irritability • Anorexia • Failure to thrive • Increased .39
pigmentation on back of hands, fingers and nose • Glossitis • Tremors and developmental
retrogression (rare) • Neurological manifestations like numbness, parasthesia, weakness,
.ataxia and diminished reflexes

Diagnosis • Peripheral blood smear • Serum vitamin B12 and folic acid assay .40
Management • Acute deficiency of vitamin B12 and folic acid can be managed by-  .41
Administration of folic acid in dosage of 2-5mg/day  Vitamin B12 administration of 1ug/
.day

APLASTIC ANEMIA .42

Definition • Aplastic anemia occurs due to marked reduction in precursor stem cells .43
.resulting in production of inadequate number of erythrocytes, leucocytes and platelets

Incidence and Etiology • It can be either hereditary or acquired. • The hereditary form of .44
Aplastic anemia is known as “Fanconi’s anemia”. • It is rare autosomal recessive disorder. •
Acquired Aplastic anemia can be either idiopathic or may result from secondary causes. •
The most common cause of Aplastic anemia is autoimmune suppression of blood cell
production. • Certain toxins and pharmacology agents implicated in the development of
Aplastic anemia are- 1. Toxic agents like benzene, insecticides 2. Pharmacological agents like
antibiotics, anti-inflammatory drugs, anticonvusltants, antimalarials, oral hypoglycemic
agents

Pathophysiology • In fetus hemapoiesis occurs in liver and spleen and after birth it .45
continues in bone marrow. • Within the marrow, the stem cells differentiate into various
types of blood cells. • Any abnormality of these stem cells leads to ‘pancytopenia’, a
.condition in which all three types of blood cells are either decreased or absent

Clinical Features • Increased bruising due to decreased platelet count • Increased .46
susceptibility to infection due to decreased WBC count • Pallor • Weakness • Breathing
.difficulty • Impaired growth

Diagnostic evaluation • History and physical examination • Complete blood picture- .47
shows pancytopenia. In severe Aplastic anemia neutrophil counts are less than 500/ul,
platelets are less than 20,000/ul and reticulocutes are less than 1% • Bone marrow biopsy:
.hypocellular marrow

Management The goal of management are- 1. to provide symptomatic treatment 2. .48

Restore bone marrow function and 3. Replace pathological bone marrow with normal tissue

i. Symptomatic treatment • If platelet count is below 10,000 cell/ul, platelet transfusions .49
are done. • If anemia is severe, packed cells are given • If infection occur due to decreased
.WBC count, antibiotics are given

ii. Restoration of bone marrow function • Androgenic steroid and corticosteroids are .50
helpful • Testosterone propionate may be given sublingually or as I/M injection • This
injection covers fatty hypocellular bone marrow into nearly normal bone marrow which
starts producing cells. • This therapy should be continued for 2-6months. • If the disease
.occurs as a result of autoimmune response, high doses of Dexamethasone may be used

iii. Bone marrow transplantation • For cases not responding to drug therapy, the .51
treatment of choice is bone marrow transplantation. • Bone marrow transplantation is done
.if histocompatible sibling is available
NURSING MANAGEMENT The aim of Nursing intervention is to prevent bleeding and .52
infection and manage problems related to anemia. A. Prevention of bleeding: i. Maintain
skin integrity and prevent pressure sores through use of water mattress or air mattress. ii.
Minimize venipuncture sites by collecting all samples at a time iii. Avoid intramuscular
injection iv. Check platelet count before any invasive procedure v. Prevent bleeding from
mucus membrane by- • Keeping mouth clean and free from debris, through use of soft brush
or mouth wash • Avoid taking rectal temperatures and administration of rectal drugs • Use
.of topical thrombin to stop bleeding from lips or nares

Contd.. B. Prevention of infection A nurse must take following actions to prevent .53
infection in the anemic child. i. Strict hand washing should be practiced by everyone who
comes in contact with the patient ii. Barrier nursing techniques by everyone who comes in
contact with child iii. Limit the number of visitors iv. Isolate the child to prevent contact with
other patients having infection

Contd… C. Management of problems associated with Anemia: i. Monitor the child’s .54
hemoglobin level on regular basis ii. Administer Oxygen if the child is dyspneic iii. Administer
packed cells if prescribed iv. Monitor growth and development of child v. Provide adequate
rest to child

THALASSEMIA .55

Introduction • The term thalassemia is derived from Greek word ‘Thalasa’ meaning ‘sea’. .56
• It is named so, because the disease had highest incidence among people living around
.Mediterranean sea like Italians, Greeks and Syrians

Definition • Thalassemia is a heterogeneous group of inherited chronic disorders, .57

characterized by absence or decreased synthesis of one or more globin chain of hemoglobin.
• It is an autosomal recessive disorder. • The patients show a variable degree of
.hypochromic anemia, with evidence of hemolysis and ineffective erythropoiesis

Types • Hemoglobin is made up of two protein chains- alpha globin and beta globin. • .58
Thalassemia occurs when there is defect in the gene that controls production of these
proteins chains. • Mainly there are two main types- I. Alpha thalassemia: it occurs when a
gene related to alpha globin protein are missing or changed II. Beta thalassemia: it occurs
when gene defect affects production of beta globin protein. It is more common than alpha
.type

Depending upon the severity thalassemia is of two types- • Thalassemia Minor: it is .59
associated with decreased beta chain synthesis and us heterozygous form of disease.
Defective gene is received form one parent • Thalassemia Major: it is also known as
‘Cooley’s anemia or Mediterranean Anemia”. It is homozygous form of disease. Defective
gene is received form both parents. Persons with thalassemia major have severe anemia and
.all the clinical manifestations of the disease

Pathologic defect • Thalassemia major is associated with little or no capacity to produce .60
b-chain of hemoglobin. • When beta chain production is absent or inadequate to meet the
physiological demands of body, marrow expands. • Large number of RBC’s are produced and
will never leave marrow • RBC’s that leave marrow will not survive for longer time. •
Marrow expansion continues leading to effective hemoglobin level, tissue anorexia and
.repeated infections. • When Hb level falls below 6gm/dl, cardiac failure occurs

Clinical features Diagnosis is usually made in 1st year of life, perhaps as early as 3 .61
months, not more commonly between 10-12 months. clinical manifestations of thalassemia
includes- • Absent or defective synthesis of hemoglobin • Inadequate structures RBC’s •
Decreased life span of RBC’s • Weakness • Exercise intolerance • Headache • Anorexia •
Precordial pain

Repeated hemolysis affects almost all organs of the body in the following manner- • .62
Skin: greenish- brown or bronze discoloration of skin. • Heart: fibrotic changes in
myocardium which leads to cardiac failure • Spleen: spleenomegaly as a result of rapid
destruction of RBC’s. • Liver: fibrosis of liver cells which progress to liver fibrosis • Pancreas:
fibrosis of pancreas leading to insulin depended DM. • Endocrine system: delayed or absent
sexual maturation • Skeletal system: thinning of cortex, widening of medullary spaces,
osteoporosis, pathological fractures leading to skeletal deformity, enlarged skull, enlarged
face, depressed nasal bridge, parietal bossing

Diagnostic evaluation I. Hemoglobin estimation II. CBP III. Peripheral Blood Smear IV. .63
Bone Marrow Examination V. Osmotic Fragility VI. Serum Bilirubin VII. Serum Iron
VIII.Radiological Studies

Management Thalassemia can’t be cured but supportive therapy is of value in .64

maintaining sufficient hemoglobin levels to prevent tissue hypoxia. Major therapeutic
modalities include- A. Transfusion therapy B. Chelation therapy C. spleenectomy

A. BLOOD TRANSFUSION • Children who cannot maintain hemoglobin level of 7g/dl, .65
should receive regular transfusions to maintain Hb levels between 10- 12g/dl. • It is
necessary to prevent chronic hypoxemia and to suppress ineffective erythropoiesis. • Group
and type specific, saline washed, packed red cell transfusions should be given to the patients
.at the rate of 10- 15ml/kg every 2-3 weeks

B. CHELATION THERAPY • Rapid hemolysis and repeated transfusions results in iron .66
overload which causes hemochromatosis and hemosiderosis. • Each unit of transfused blood
provides 200mg of elemental iron. • This excess iron deposits in various organs leading to
multiple organ failure • Chelating therapy is used to excrete excessive iron in urine and
creates a negative iron balance in body. • The iron status of child should be monitored by
repeated serum ferretin measurements. • DESFERRIOXAMINE is available as iron chelating
agent that may be given Parenteral route • It should be given subcutaneously in dose of 40-
60mg/kg/ day over a period of 8-12hrs. • This therapy should be started from 10th to 15th
.transfusions

C. SPLEENECTOMY • The spleen acts as a store for non-toxic iron, protecting the body .67
form extra iron, thus early removal of spleen may be harmful. • Spleenectomy is justified
only in patients with hyperspleenism, leading to excessive destruction of erythrocytes and
increased need for frequent blood transfusion which results in further iron accumulation •
This procedure should be considered for the patients who require more than 200-250ml/kg
.of packed cells annually

NURSING MANAGEMENT I. Early assessment II. Preparation of child for diagnostic .68
procedures III. Care during blood transfusion IV. Administration of chelation therapy V.
Prevention of infection VI. Education and support of parents and child

Care during blood transfusion • Blood grouping and cross matching should be ensured .69
before transfusing the blood • Blood should be screened for blood borne disorders •
Observe the patient for transfusion reaction causing child, itching, rash, headache and back
pain. • Always pre-warm the blood before administration • Monitor vital signs during blood
.transfusion • Infuse blood slowly to prevent blood overload • Follow strict aseptic principles

Prognosis • Outcome depends on the severity of disease. • If the child has thalassemia .70
major, he requires frequent blood transfusion and chelation therapy • With chelation
therapy child can survive up to 30 years • Death usually occurs due to cardiac complications
.during 2nd decade of life

Prevention • Thalassemia screening should be done to find out carrier status of .71
individuals who are planning to get married as they may give birth to the same children. • It
can be prevented by marital counseling, prenatal diagnostics and abortion of affected fetus
.among thalassemia parents

HEMOPHILIA .72

DEFINITION • Hemophilia is genetically transmitted blood clotting disorder, caused by .73

.deficiency of coagulation factor VIII (antihemophilic factor) or factor IX (christmas factor)

Types • Depending on the clotting factor involved, hemophilia is of following types: i. .74
Hemophilia A or Classic hemophilia: it occurs due to deficiency of clotting factor VIII. It is
most common form of disorder present in about 1 in 5000-10000 male births ii. Hemophilia
B or christmas disease: it occurs due to deficiency of clotting factor IX. It occurs in around 1
in 20000-34000 male births Other less common types are- iii. Hemophilia C: it occurs due to
lack of factor XI IV. Hageman’s disease: it occurs due to deficiency of factor XII V. Von
Willebrand’s Disease: it is caused by reduced level of von willebrands factor, a multi proterin
thet binds with factor VIII nad protects it form rapid breakdown within blood

Transmission of disease • Hemophilia A, B are inherited as sex linked (x) linked recessive .75
disorder. • Hemophilia is more likely to occur in males than females • This is because
females have 2 x chromosomes while males have only one x chromosome, so defective gene
is guaranteed to manifest in any male who carries it. • Females are always asymptomatic
carries f this disorder • Hemophilia C is an autosomal genetic disorder which can affect both
.sexes

Pathophysiology • An inherited deficiency of factor VIII or IX alters the activation of .76

intrinsic clotting pathway. • As a result children with hemophilia A or B shows manifestations
of prolonged bleeding or delayed clotting. • The severity of manifestations is proportionate
to the severity of factor deficiency. • The normal level of factor VIII is 60-140% and of factor
IX is 60- 145%. • A factor level of 30% or less is diagnostic of hemophilia. • Depending on
level of factor deficiency, hemophilia is classified may be classified as- 1. Mild hemophilia:
the level of factor VIII or OIX is 6- 30% 2. Moderate hemophilia: the level of factor VIII or IX is
.2-5 % 3. Severe hemophilia: the level of factor VIII or IX is 1% or less

Clinical features • Mild hemophilia: a. Frequent bruises b. Nose bleeds c. Bleeding gums .77
d. Prolonged bleeding after minor surgeries • Moderate hemophilia: a. Frequent bleeding
episodes b. Joint bleeding • severe hemophilia: a. Bleeding as early as on 1st day of life b.
Excess bleeding after injections c. Spointaneous bleeding into joint cavities d. Subcutaneous
.and intramuscular hemorrhages e. Intracranial hemorrhages

Diagnostic evaluation • History and clinical features • Genetic history • Labaratory tests .78

Management • Replacements preparations of factor VIII and IX that are available as .79
cryoprecipitate made from fresh plasma. One unit of plasma provides 75-125 units of
coagulation factors. • Fresh frozen plasma can also be used to correct. Each ml of plasma
contains 1 unit of factor VIII and slightly less than 1 unit f factor IX. • Desmopressin: this drug
may be used to treat hemophilia. It works by increasing the amount of clotting factor VIII in
.child’s blood

Nursing management A. Prevention of bleeding B. Control bleeding C. Administration of .80

.replacement of therapy D. Education and support to patient and family

Prevention of bleeding • Shorten child’s finger nails to aviod scratching • Avoid using .81
diaper pins in infants • Provide soft toys whithout sharp corners • Child should be given soft
tooth brush

Control of bleeding • First aid measures such as- R- rest: make the child to lie down .82
quietly until bleeding stops I- ice: apply an ice pack on the bleeding area. C- compress: apply
pressure over the bleeding site. A bleeding joint may be wrapped with an elastic
compression bandage. E-elevate: position the child so that the bleeding area is raised. Raise
.the area above the level of heart if possibel

Prognosis • With the advent of factor replacement therapy and with proper care and .83
support, hemophilics can lead a long and productive life. • Hemophilics now a days were
.reaching adulthood with minimal side effects

PURPURA .84

DEFINITION • Purpura is a bleeding disorder characterized by petechiae and ecchymosis .85

which may be due to either deficiency in number or quality of platelets or defect in vascular
.endothelium. Purpura occurs due to bleeding from capillaries, under the skin

Types • Purpura is of 2 types- 1. IDIOPATHIC THROMBOCYTIC PURPURA 2. .86

ANAPHYLACTOID PURPUS

IDIOPATHIC THROMBOCYTOPENIC PURPURA .87

Defintion • Idiopathic thrombocytopenic purpura, the most common acquired disorder .88
in children which is hemorrhagic involving skin, mucus membrane and internal organs. • It is
most likely an autoimmune response of the body. • Its greatest incidence is seen between 3-
.7 years of age

Pathophysiology • Normally the platelets are formed from Megakaryocytes in bone .89
marrow. • In 60 % cases of Purpura, the cause is autoimmune, with antibodies against
platelets. • Most often these antibodies are against platelet membrane glycoprotein and are
.immunoglobulin type. • These antibodies destroy platelets leading to platelet deficiency

Clinicial feature The child with Purpura may present with- • Petechiae • Ecchymosis • .90
Bleeding form gums • Epistaxis • Anemia • Internal hemorrhage • Hemarthrosis • Low grade
fever • Intracranial or intracerebral hemorrhage

Diagnostic evaluation • Platelet count: it will be below 20,000/mmcube (1.5-4.5 .91

lakhs/mmcube is normal) • Bleeding time and clotting time increased • Peripheral blood
smear- giant forms and stained platelets are seen • Bone marrow examination: abundant
.platelet precursors are found

Management • Steroids • Anti D antibodies • Steroid sparing agents • Immunoglobulin's .92

• Platelet infusion

ANAPHYLACTOID PURPURA .93

DEFINTION • Anaphylactoid pupura is a systemic inflammatory disorder, seen primarily .94

.in children between 2-8 years of age. Boys are affected twice as frequently as girls

Etiology • Recent upper respiratory infection history • Exact etiology is unknown • .95
Possible precipitating factors include- 1. Drugs like penicillin, aspirin 2. Foods 3. Insect bites
.4. Bacterial or viral infections

Clinical features • Erythematous petechiae rashes appearing symmetrically over .96

buttocks and extremities • GI symptoms include- colicky abdominal pain, vomiting.
Intussusceptions • Synovial membrane vasculitis • Renal manifestations like- hematuria,
proteinurina, hypertension

Diagnostic evaluation • Diagnosed based on typical skin rash • Joint and renal findings .97
also helps to diagnosis

Management • Antibiotics are given for specific bacterial infections • Short term steroid .98
.therapy • Analgesics like acetaminophen to relieve pain

Nursing management • Minimize chances of trauma to the child and provide safe .99
environment • Control bleeding • Administer prescribed drugs

Prognosis • The prognosis of purpura is good if adequate supportive care is given and .100
.complications are prevented. • Most of the patients recover within 2-3 months of onset

…THANK YOU .101

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